Summary about Disease
Felty's syndrome is a rare disorder characterized by the combination of three conditions: rheumatoid arthritis (RA), an enlarged spleen (splenomegaly), and a low white blood cell count (neutropenia), which increases the risk of infection. It is more common in people who have had RA for a long time.
Symptoms
Joint pain and inflammation (typical of rheumatoid arthritis)
Fatigue
Enlarged spleen (may cause abdominal discomfort)
Recurrent infections (due to low white blood cell count, particularly neutropenia)
Skin ulcers, especially on the legs
Anemia (low red blood cell count)
Thrombocytopenia (low platelet count)
Weight loss
Eye inflammation (episcleritis or scleritis)
Causes
The exact cause of Felty's syndrome is unknown, but it is thought to be an autoimmune disorder associated with long-standing rheumatoid arthritis. Genetic factors and environmental triggers are believed to play a role. The body's immune system mistakenly attacks its own tissues, leading to inflammation in the joints, spleen enlargement, and destruction of neutrophils in the bone marrow and spleen.
Medicine Used
Disease-modifying antirheumatic drugs (DMARDs): Methotrexate, sulfasalazine, leflunomide, and hydroxychloroquine are commonly used to control the underlying rheumatoid arthritis.
Biologic agents: TNF inhibitors (e.g., etanercept, infliximab, adalimumab), rituximab, abatacept, and tocilizumab may be used to reduce inflammation and improve blood counts.
Granulocyte-colony stimulating factors (G-CSF): Filgrastim and pegfilgrastim can help increase the white blood cell count and reduce the risk of infection.
Corticosteroids: Prednisone may be used to reduce inflammation in severe cases, but long-term use is generally avoided due to side effects.
Antibiotics: Used to treat infections as they arise.
Is Communicable
No, Felty's syndrome is not communicable or contagious. It is an autoimmune disorder, not an infectious disease.
Precautions
Preventing infection: Frequent handwashing, avoiding contact with sick people, getting vaccinated against influenza and pneumonia.
Managing RA: Following prescribed medication regimen, regular monitoring by a rheumatologist.
Skin care: Protecting skin from injury, keeping skin clean and moisturized, especially on the legs.
Regular blood tests: To monitor white blood cell count and liver function.
How long does an outbreak last?
Felty's syndrome is a chronic condition, not an "outbreak." Symptoms fluctuate, but the underlying disorder persists. Flares of arthritis and infections may occur intermittently. The duration of these flares varies depending on treatment and individual factors.
How is it diagnosed?
Diagnosis involves:
Medical history and physical examination: Assessing symptoms and signs, especially history of rheumatoid arthritis.
Blood tests: Complete blood count (CBC) to check for neutropenia, anemia, and thrombocytopenia; rheumatoid factor (RF) and anti-citrullinated protein antibody (ACPA) tests; antinuclear antibody (ANA) test.
Spleen imaging: Ultrasound, CT scan, or MRI to assess spleen size.
Bone marrow biopsy: May be performed to evaluate the production of blood cells.
Timeline of Symptoms
The timeline of symptoms varies greatly among individuals. Generally:
Years before diagnosis: Patient has established rheumatoid arthritis.
Onset of Felty's: Gradual development of neutropenia and enlarged spleen.
Later stages: Recurrent infections, skin ulcers, and other complications may arise.
Important Considerations
Increased risk of infection: Neutropenia makes individuals more susceptible to bacterial, viral, and fungal infections.
Potential for serious complications: Untreated infections can lead to sepsis. Skin ulcers can be difficult to heal.
Treatment goals: Reduce inflammation, improve blood counts, and prevent infections.
Multidisciplinary approach: Requires collaboration between rheumatologists, hematologists, and infectious disease specialists.
Monitoring for side effects: DMARDs and biologic agents can have significant side effects that need to be monitored.